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Successfully treated Hodgkin's disease followed by mycosis fungoides: case report and review of the literature.

We report the case of a 30-year-old man who had Hodgkin's disease of the nodular sclerosing type and subsequently developed mycosis fungoides. The Hodgkin's disease was treated with radiation therapy and chemotherapy, and the patient was in complete remission. Seven years later mycosis fungoides occurred and rapidly became progressive. Autopsy revealed that the mycosis fungoides involved multiple organs without any evidence of Hodgkin's disease. The possible significance of the association of these two diseases is presented.

Adult↗

Sclerodactyly in a patient with mycosis fungoides.

A 44-year-old man had mycosis fungoides and generalized plaque disease involving 80% of his skin surface with diffuse lymphadenopathy and alopecia of the scalp and groin. In addition, distal to the wrist, there were sclerodermatous changes involving the skin of the hands with associated sclerodactyly of all digits with loss of normal palmar creases. There were no subungual telangiectasis or digital ulcers. The changes in the hand that occurred in this case, no doubt arose as a result of the patient's neoplasm. Abnormalities of collagen biosynthesis and degradation probably occur with mycosis fungoides as a result of the extensive infiltration of the epidermis and dermis with malignant cells. To our knowledge, the association of sclerodactyly with mycosis fungoides has not been previously reported.

Adult↗

Mycosis fungoides-like lesions associated with phenytoin therapy.

An 83-year-old woman developed generalized lymph node hyperplasia together with mycosis fungoides-like skin lesions 11 months after institution of anticonvulsant therapy with phenytoin. The clinicopathological changes disappeared completely three weeks after cessation of therapy. This circumstantial evidence together with the known data on the association of phenytoin with lymphoproliferative disorders suggest that it was the phenytoin that was responsible for the patient's condition. This case had features similar to those associated with the pseudo-mycosis fungoides syndrome except that the cutaneous lesions consisted of two localized erythematous plaques, with no generalized exfoliative erythrodermic dermatitis. Nonetheless, we believe that the use of the term pseudo-mycosis fungoides for this and similar cases would be appropriate.

Aged↗

Topical chemotherapy of mycosis fungoides.

Mycosis fungoides is a T-cell lymphoma which is often localized to the skin in the early stages. Untreated, the process eventually progresses through eczematous, plaque, and tumor stages to systemic involvement. Its course, however, is unpredictable. Topical chemotherapy is effective in early stages of mycosis fungoides. Possibly prognostic benefits can occur from the early use of these agents. Nitrogen mustard and BCNU, both alkylating agents, have been used topically to control the disease. A dermatitis may develop in persons treated with nitrogen mustard but systemic side-effects are rare. However, BCNU may rarely lead to marrow depression when used topically. The use of these agents in mycosis fungoides is discussed herein.

Administration, Topical↗

T- and B-cells and IgE in mycosis fungoides.

The role of T- and B-lymphocytes and serum IgE was studied in 22 patients with mycosis fungoides. The distribution of B-cells in peripheral blood was normal, while the mean percentage of T-cells was significantly lower than in 14 healthy controls. Four patients with mycosis fungoides in stages I to IV had a highly elevated serum IgE, while serum IgE in remaining patients was slightly elevated, normal, or subnormal. The mean serum IgE level was not significantly elevated. Our results tend to show that a reduced ability to react with cellular immunity may be an important factor in mycosis fungoides. This may have therapeutic aspects.

Adolescent↗

Lymphography in the assessment of mycosis fungoides.

Extracutaneous manifestations of mycosis fungoides imply a bad prognosis and are a major cause of death. Benign dermatopathic lymphadenopathy is associated with mycosis fungoides and often precedes lymphomatous infiltration. In this study, 10 patients in the early stages of mycosis fungoides underwent clinical and lymphographical examinations. In one the lymphoma was already present in lymph nodes. Six had signs of dermatopathic adenopathy which was verified by lymph node biopsy in 5. In one of these the disease later progressed to a malignant lymphoma. The frequent occurrence of lymph node involvement justifies the use of lymphography collaterally with staging laparotomy to determine the presence of pathologic retroperitoneal lymph nodes.

Adult↗

Results of lymphography in early mycosis fungoides.

Lymphography was performed in 28 patients with mycosis fungoides. In 22 of the patients, the investigation took place prior to 2 months after the diagnosis was established, and in 7 of these lymphography was made before the histological verification of mycosis fungoides was possible. Five patients with widespread, persistent and severe atopic dermatitis served as controls. Eighteen patients with mycosis fungoides (64%) had abnormal lymphograms, while all 5 controls had normal lymphograms. Abnormal findings were diagnosed in 12 of 22 patients at the earliest time possible during the course of their disease and even found in 5 of 7 patients who only had premycotic lesions at the time of investigation. These results may have some bearing on therapy, suggesting that systemic treatment could possibly be introduced at a far earlier disease stage than is the custom at present.

Dermatitis, Atopic↗

[Association between mycosis fungoides and myasthenia gravis (author's transl)].

Myasthenia gravis characterized by physical examination, electrophysiologic abnormalities, ephedronium test, without thymoma, associated in a 47 year-old-man, with mycosis fungoides. Myasthenia gravis began two years after the onset of mycosis fungoides, and the patient died of infection four years after the development of the cutaneous lymphoma. At autopsy, there was no visceral involvement. Myasthenia gravis and lymphoma rarely coexist; the occurrence of myasthenia gravis shortly after the onset of mycosis fungoides suggests that the two disorders may be linked by immunological abnormalities.

Autoantibodies↗

Mycosis fungoides involving the brain and optic nerves.

In a patient with long-standing plaque-stage mycosis fungoides, eye pain, a visual field defect, decreased acuity, and a swollen optic disc developed. A lymphomatous infiltrate consistent with mycosis fungoides was found in the brain at craniotomy and in the optic nerves at autopsy. Ten months elapsed between the presenting symptoms and death and during this period the patient's symptoms were controlled with prednisone therapy. The optic nerves as well as the brain can be involved with mycosis fungoides.

Brain↗

Mycosis fungoides cells in sputum. A case report.

Atypical lymphoid cells were identified in the sputum of a patient known to have mycosis fungoides. Although the lungs are the second most common organ to be affected when there is extracutaneous dissemination of mycosis fungoides, this is the first report of pulmonary involvement diagnosed by the cytologic identification of "mycosis cells" in the sputum.

Adult↗

Subcutaneous mycosis fungoides.

In five cases of mycosis fungoides, previously treated with electron-beam therapy, subcutaneous nodules developed. Clinically, these lesions were thought to be epidermoid cysts or lipomas, but on biopsy were discovered to be subcutaneous infiltrates, three of which were diagnosed as mycosis fungoides. The other two specimens showed only a nonspecific subcutaneous infiltrate. There is no ready explanation for the appearance of these lesions, but it is speculated that they may be the result of inadequate penetration of the electron beam to the depth at which some atypical cells may originally have been located. Patients with mycosis fungoides who develop unusual subcutaneous nodules should be fully investigated so that appropriate and adequate therapy may be initiated.

Adult↗

[Morphologic features of tumor stage lesions of mycosis fungoïdes (author's transl)].

A morphological analysis was carried out on a series of five patients, with tumor stage lesions of mycosis fungoides. The histological findings of skin, lymph nodes, spleen, liver and bone marrow were investigated. Histologically the cutaneous infiltrate consisted of large cells (mycosis cells) and small atypical lymphoid cells (Lutzner cells). Among the large cells, which predominated in number, two principal types of cell could be distinguished: lymphoid cells comparable to the Lutzner cells because of their nuclear configuration, and poorly differentiated basophilic and pyroninophilic cells with irregular nucleus ("reticulum" cells?) or regular nucleus ("T-immunoblasts"?). Ultrastructural data of cutaneous lesion obtained for study from only one patient, were consistent with these findings. Lesions of lymph nodes and viscera consisted of a cellular composition which closely resembled that of cutaneous lesions. In particular, the large so-called mycosis cells were always found and represented a supportive histologic feature in identifying this disease.

Adult↗

[High-grade malignant mycosis fungoides with cytological transformation (author's transl)].

In the Kiel-classification of non-Hodgkin lymphomas a distinction is made between low and high grade malignancy. Mycosis fungoides is listed among the low grade malignant lymphomas because in the cellular infiltrate small lymphoid cells are predominant. Two cases are reported in which the cellular infiltrate of the cutaneous lesions consists mostly of large lymphoid cells resembling lymphoblasts and immunoblasts indicating a high grade malignancy. This type of cellular infiltrate appeared as a blastic transformation from mycosis fungoides very early in one case and at a later stage in another. Clinically these two cases were characterized by a rapid progression leading to the deaths of the patients. The cytological analysis in cutaneous lesions of mycosis fungoides seems to have a prognostical and possibly a therapeutical bearing.

Adult↗

[Change of level of mycosis fungoides under systemic PUVA-therapy (author's transl)].

In a patient with mycosis fungoides the typical skin lesions showed a good response to oral photochemotherapy (PUVA), which could be confirmed by serial control biopsies. Twelve months after onset of therapy large subcutaneous nodules appeared, whereas the upper skin remained uninvolved. Two months later the patient died with disseminated visceral involvement, whereas skin lesions were minimal. The case reported here shows that improvement of the skin lesions of mycosis fungoides under systemic PUVA-therapy can lead to misjudgement of the actual course of the disease. PUVA-therapy of mycosis fungoides may cause a change of the involved layers, leading from the cutis to the subcutis, i.e. disappearance of epidermotropic plaque-like infiltrates and appearance of subcutaneous nodules and visceral involvement.

Aged↗

Dermatopathic lymphadenopathy. Comparison of cases associated and unassociated with mycosis fungoides.

Thirty-three biopsies showing dermatopathic lymphadenopathy were obtained from patients with documented cutaneous mycosis fungoides and were studied together with an equal number of dermatopathic lymph nodes derived from patients without evidence of mycosis fungoides. The nodes were evaluated for a variety of histologic features including mitotic figures, immunoblasts, and in particular for the number of atypical cerebriform lymphocytes. Atypical lymphocytes were found to be equally as frequent among both groups of dermatopathic lymph nodes without any statistically significant differences in quantitation or distribution. Similarly, no other morphologic variable was found which would allow an objective distinction of dermatopathic lymphadenopathy from patients with or without mycosis fungoides.

Adolescent↗

Woringer-Kolopp disease (localized pagetoid reticulosis) or unilesional mycosis fungoides? An analysis of eight cases with benign disease.

BACKGROUND: The controversial nosology of Woringer-Kolopp disease (localized pagetoid reticulosis, unilesional mycosis fungoides) is being clarified by the systematic immunophenotypic and immunogenetic examination of infiltrating lesional T lymphocytes. The clinical course and immunohistochemical characteristics of eight cases of Woringer-Kolopp disease are described. OBSERVATIONS: Lesions measured 0.8 x 0.5 to 16.0 x 15.0 cm. Histologically, all cases resembled mycosis fungoides-type cutaneous T-cell lymphoma and phenotypic analysis supported their designation as an epidermotropic T-cell process. Phenotypic aberrancy was not noted on immunohistochemical analysis of paraffin-embedded tissue. Three of four patients with available fresh-frozen tissue specimens demonstrated reduced or absent expression of CD7 (Leu-9) and/or Leu-8, while loss of the pan-T-cell markers CD2, CD3, and CD5 was not observed. Only in half these patients was a lesional predominance of CD4+ T-cells revealed. Germline DNA was detected in a lesional skin specimen obtained from one patient tested for T-cell receptor gene rearrangements. After treatment, the observation of disease-free periods ranging from 18 months to 17 years (mean, 5.9 years) reinforces the view that Woringer-Kolopp disease is a focal pathologic event with a favorable prognosis. No patient experienced a local recurrence or distant spread of the disease. CONCLUSION: This and previous studies suggest that Woringer-Kolopp disease is a unique, benign unilesional T-cell lymphoproliferative process with certain histologic and phenotypic similarities to both early epidemotropic mycosis fungoides-type cutaneous T-cell lymphoma and other T-cell lymphoproliferations.

Adult↗

The treatment of mycosis fungoides and related conditions with particular emphasis on electron therapy.

The difficulties created by the lack of generally accepted views on classification, diagnostic criteria and natural history of the cutaneous lymphomata are discussed. Only the lymphomata which remain confined to the skin throughout the major part of their development are considered. It is emphasized that these are extremely radiosensitive and that there must be some factors as yet unknown which play a part in their unique natural history and response to treatment. The cutaneous lymphomata are considered under three headings: (1) lymphocytoma, (2) lymphosarcoma and reticulum cell sarcoma, (3) mycosis fungoides. The characteristics of the first two are discussed briefly and illustrative examples are given. The relatively benign nature of the lymphocytoma is stressed. It is suggested that the solitary lymphosarcoma of the skin should be treated by radical radiotherapy since long-term survival or cure may be achieved. The treatment of mycosis fungoides is considered in greater detail. The physical advantages of electron therapy are described and details given of whole body surface treatment with electrons produced by a 6 MeV linear accelerator and slowed down with carbon decelerators of appropriate thickness. The results in 73 patients treated by electrons are analysed. The early and late side-effects are mentioned and the necessity for investigating other forms of treatment is stressed. It is concluded that at the present time electron therapy is the most satisfactory form of treatment for mycosis fungoides.

Adult↗